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Os odontoideum and craniovertebral junction instability secondary to dystonia: case series and review of the literature

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Abstract

Introduction

Os odontoideum refers to a rounded ossicle detached from a hypoplastic odontoid process at the body of the axis. The aetiology has been debated and believed to be either congenital or acquired (resulting from trauma). Os odontoideum results in incompetence of the transverse ligament and thus predisposes to atlantoaxial instability and spinal cord injury.

Methods/results

Three cases of children with severe dystonic cerebral palsy presenting with myelopathic deterioration secondary to atlantoaxial instability due to os odontoideum are presented. This observation supports the hypothesis of os odontoideum being an acquired phenomenon, secondary to chronic excessive movement with damage to the develo** odontoid process.

Conclusion

In children with cerebral palsy and dystonia, pre-existing motor deficits may conceal an evolving myelopathy and result in delayed diagnosis of clinically significant atlantoaxial subluxation.

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Acknowledgements

We recognize all the nurses and trainees who have diligently documented the data for the patients included in this study. We have no financial disclosures.

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Authors and Affiliations

  1. Great Ormond Street Hospital for Sick Children, London, England

    Michelle Kameda-Smith, Asthik Biswas, Felice D’Arco & Dominic Thompson

  2. Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, London, England

    Michelle Kameda-Smith & Dominic Thompson

  3. Department of Paediatric Neuroradiology, Great Ormond Street Hospital for Children, London, England

    Asthik Biswas & Felice D’Arco

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  1. Michelle Kameda-Smith
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Contributions

DT conceptualized the study. MKS and DT extracted the data. MKS and DT wrote and critically revised the manuscript. Manuscript reviewed by AB, FD and DT. Project supervised by DT.

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Correspondence to Dominic Thompson.

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Kameda-Smith, M., Biswas, A., D’Arco, F. et al. Os odontoideum and craniovertebral junction instability secondary to dystonia: case series and review of the literature. Eur Spine J 33, 1164–1170 (2024). https://doi.org/10.1007/s00586-023-08044-1

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